Download Inherited Metabolic Disease Benefit List

INHERITED METABOLIC DISEASE BENEFITS
Select formula, low protein food and drugs may be included as an Inherited Metabolic
Disease Benefit for clients who meet the established criteria.
A. Formula and Low Protein Food
In addition to being an eligible Saskatchewan Health beneficiary, clients must:
1. be diagnosed with an inherited metabolic condition (may or may not be
screened through newborn screening),
2. be prescribed treatment that includes formula that is used to replace an element
of a typical diet (e.g. protein),
3. be prescribed formula that is only a component of their diet and is/will be less
than 90% of their caloric intake after age one,
4. be prescribed formula that is not available through local suppliers; and,
5. follow a strict diet. If the diet is not followed, symptoms will be severe and
include, but are not limited to, cognitive impairment and/or physical disability
such as blindness.
Eligible clients receive the following benefits at no charge:
1. Formula from the list of approved products.*
2. Low protein food from the list of approved products.*
* The legislative authority for the formula and the low protein food is The Saskatchewan Aids to
Independent Living Regulations, 1976 (Saskatchewan Regulation 292/76).
1. Formula
FORMULA
Abbott
Nutricia (continued)
Cyclinex 1
Cyclinex 2
400g
400g
Glutarade Junior GA-1 Drink Mix
MSUD Aid
400g
200g
Glutarex-1
Glutarex-2
400g
400g
MSUD Analog
MSUD Maxamaid
400g
454g
Hominex-1
Hominex-2
400g
400g
MSUD Maxamum
Periflex Advance
454g
454g
I-Valex 1
I-Valex 2
400g
400g
Periflex Infant
Periflex Junior
400g
454g
Ketonex-1
Ketonex-2
400g
400g
Periflex Junior Plus
Phenylade 60
400g
454g
Phenex 1
Phenex 2
400g
400g
Phenylade
Phenylade Amino Acid Blend
454g
454g
Pro-Phree
Propimex 1
400g
400g
Phenylade Essential
Phenylade GMP
454g
400g
Propimex 2
Tyrex-1
400g
400g
Phenylade MTE Amino Acid Blend
TYR Anamix Next
454g
400g
Tyrex-2
Cambrooke
400g
UCD Anamix Junior
XLYS Xtrp Analog
400g
400g
XLYS Xtrp Maxamaid
XLYS Xtrp Maxamum
454g
454g
400g
454g
Bettermilk
Camino Pro PKU
Mead Johnson
49g-52g
140ml
BCAD-1
454g
XMET Analog
XMET Maxamaid
BCAD-2
PFD-1
454g
454g
XMET Maxamum
XMTVI Analog
454g
454g
PFD-2
Phenyl-Free 1
454g
454g
XMTVI Maxamaid
XMTVI Maxamum
454g
454g
Phenyl-Free 2
Phenyl-Free 2 HP
454g
454g
XPhe Maxamaid
XPhe Maxamum
454g
454g
Tyros-1
Tyros-2
Nutricia
454g
454g
XPHE Xtyr Analog
XPHE Xtyr Maxamaid
400g
454g
Acerflex
454g
XPHEN TYR Maxamaid
XPHEN TYR Maxamum
500g
500g
Complex Essential MSD Drink Mix
Complex Junior MSD Drink Mix
454g
400g
XPTM Analog
Vitaflo
400g
Complex MSUD Amino Acid Blend
Duocal
454g
400g
EAA Supplement
HCU Cooler
12.5g
130ml
Glutarade Essential
Glutarade GA-1 AA Blend
454g
454g
HCU Cooler 20
HCU Express
174ml
25g
2. Low Protein Food
LOW PROTEIN FOOD
Baking Mixes
Breads
Aproten Flour Mix (Farina)
Cambrooke Baking Mix
Cambrooke Mix Quick
Cambrooke Wheat Starch
Country Sunrise Muffin Mix
Country Sunrise Pancake Mix
Dietary Specialties Bread Machine Mix
Dietary Specialties Low Protein Baking Mix
Juvela Low Protein Mix
Nutricia Loprofin All Purpose Baking Mix
Nutricia Maddy's Homestyle Muffin Mix
Nutricia Wel-Plan Wheat and Corn Starch
Baking Mix
Taste Connections Low Protein Bread Mix
Taste Connections Low Protein Versa Mix
Taste Connections Multi-Baking Mix
Taste Connections Wheat Starch
Rice
Aproten Chicchi/Riso Rice
Cambrooke Short Grain Rice
Dietary Specialties Imitation Rice
Nutricia Loprofin Rice
Promin Pasta Cous Cous
Promin Pasta Imitation Rice
Meat Substitutes and Cheese
Cambrooke Brookelyn Dogs
Cambrooke Camburgers
Cambrooke Cheese Shreds
Cambrooke Cheese Singles
Cambrooke EGGZ
Cambrooke GO! Pockets
Cambrooke Meatballs
Cambrooke Mini Pockets
Cambrooke Pierogi
Cambrooke Artisan Bread
Cambrooke Bagels
Cambrooke Breakfast Bars
Cambrooke Brookelyn Dog Buns
Cambrooke Camburger Buns
Cambrooke Cinnamon Raisin Swirl Bread
Cambrooke Homestyle Sliced White Bread
Cambrooke Pita Pockets
Cambrooke Scones
Cambrooke Southwestern Biscuits
Cambrooke The Bigger Bagels
Cambrooke Tortilla Wraps
Cambrooke Ravioli
Canbrands PaneRiso Egg Replacer
Country Sunrise Burger Mix
Country Sunrise Imitation Scrambled Egg and
Omelette Mix
Country Sunrise Peanut Butter Spread Mix
Country Sunrise Vegetable Chicken Nugget Mix
Country Sunrise Vegetable Hot Dog Mix
Dietary Specialties Peanut Butter Flavoured
Spread
Ener-G Egg Replacer
Walden Farms Whipped Peanut Spread
Cambrooke Tuscan Pizza Crusts
Cereals
Dietary Specialties Porridge
Nutricia Loprofin Breakfast Cereal Loops
Nutricia Milupa LP-Flakes
Nutricia Milupa LP-Fruity Cereal Mix
Promin Pasta Meal (Porridge)
Pastas
Aproten Pasta, Assorted
Cambrooke Pasta, Assorted
Dietary Specialties Pasta, Assorted
Nutricia Loprofin Pasta, Assorted
Promin Pasta, Assorted
Snacks
Aproten Biscotto
Aproten Cracker Toast/Fette Tostate
Aproten Crispbread/Rusks
Nutricia Loprofin Crackers
Miscellaneous
Cambrooke Alfredo Sauce Mix
Cambrooke Chicken Consomme and
Seasoning
Cambrooke Shake N'Cheese
Country Sunrise Cheese Flavoured Sauce
Mix
Country Sunrise Instant Mashed Potato Mix
Dietary Specialties Cheese Flavoured Sauce
Mix
Dietary Specialties Tomato Flavoured Sauce
Mix
La Tiara Taco Shells
Nutricia Milupa LP-Drink Mix
Washington Golden Seasoning and Broth
Washington Rich Brown Seasoning and Broth
B. Drug Benefits
Please note, physicians may make individual case by case requests for medications to
treat inherited metabolic diseases. Requesting physicians are asked to provide the Drug
Plan with clinical information to support the request.
1. saproterin dihydrochloride (Kuvan-BPC) - Criteria For Coverage
For more information on how to obtain coverage, or if you have questions regarding
the criteria or billing procedures, please contact the Drug Plan.
Initial Inclusion Criteria:
Non-Pregnant Patients and Patients actively planning pregnancy:

For the management of patients with the diagnosis of Phenylketonuria (PKU)
who meet ALL of the following criteria:
1.
2.
Compliance with low protein diet and formulas.
Baseline blood phenylalanine (Phe) levels > 360 μmol/L despite
compliance with low protein diet (require at least 2 levels during 3 to 6
month time frame).
3. Baseline protein intake assessment by a dietitian.
4. Ability to comply with medication regimen.
5. Managed by a physician specialized in metabolic/biochemical
diseases.
Pregnant Patients:

For the management of patients with the diagnosis of Phenylketonuria (PKU)
who meet ALL of the following criteria:
1.
2.
Managed by a physician specialized in metabolic/biochemical
diseases.
Baseline blood phenylalanine (Phe) levels > 360 μmol/L despite
compliance with all recommendations for dietary intervention and
monitoring.
Exclusion Criteria:
•
Known hypersensitivity to sapropterin or its excipients.
•
Any other contraindications.
•
Baseline Phe Levels <360 μmol/L for non-pregnant patients.
•
Baseline Phe Levels <360 μmol/L for pregnant patients.
•
Women who are nursing/breast feeding.
•
Patients not on diet or not compliant with diet.
Caution:
•
Taking medication known to inhibit folate synthesis (e.g., methotrexate).
•
Any condition requiring treatment with levodopa or any PDE-5 inhibitor (e.g.,
sildenafil).
Test for Eligibility: 72 hour Kuvan Challenge


72 hour challenge with Kuvan at 20 mg/kg/day - blood Phe concentrations are
measured at the following timepoints (in hours): T -48 to T -24, T0, T4 to T12, T
24, T 48 to T 72, OR
As per clinic’s protocol.
Dose of Kuvan to establish clinical benefit is 20 mg/kg/day.
Definition of Responder to Kuvan Challenge:
For Non-Pregnant Patients and Patients actively planning pregnancy:
Criteria to determine if patients have “responded” to 72 hour Kuvan challenge:


Reduction in Phe blood level of at least 30% compared to baseline; AND
Baseline assessment of neurobehavioural or neurocognitive impairment* and
quality of life assessment due to PKU after 72 hr Kuvan challenge but before
start of Kuvan therapy (this assessment does not apply to pregnant women).
Note:

Baseline Phe tolerance level must be documented as well as Phe tolerance
levels documented at months 1-2 and 4-6 while on Kuvan therapy.
Approval Duration: 6 months (KUVAN THERAPY TO BE FUNDED BY THE
MANUFACTURER FOR 6 MONTHS)
* For children less than 4 years of age, clinically validated age-appropriate
neurobehavioural, neurocognitive, or developmental tests may be selected at the
clinician’s discretion rather than PKU specific tests.
Pregnant Patients:
Criteria to determine if patients have “responded” to 72 hour Kuvan challenge:
1.
Reduction in Phe blood level of at least 30% compared to baseline
after 72 hours.
For patients who meet “responder” definition to Kuvan Challenge, may be eligible for
Kuvan funding if the following criteria are met:

A decrease in Phe concentration to less than 360 μmol/L to be maintained for
the duration of pregnancy to be eligible for continued funding.
Approval Duration: Until end of pregnancy, no renewals (KUVAN THERAPY TO BE
FUNDED BY THE MANUFACTURER FOR 6 MONTHS)
INITIAL REQUESTS FOR FUNDING AND RENEWAL REQUESTS:
(AFTER INITIAL SIX-MONTH TRIAL DEMONSTRATING RESPONSE TO KUVAN
THERAPY)
Non-Pregnant Patients and Patients actively planning pregnancy:

For the management of patients with the diagnosis of Phenylketonuria (PKU)
who meet ALL of the following criteria:
1. Compliance with low protein diet, formulas, and Kuvan; AND
2. Achieve:
a) normal sustained Blood Phe levels [ > 120 μmol/L and <
360 μmol/L] (At least 2 levels measured at least 1 month
apart); OR
b) sustained blood Phe reduction of at least 30% (At least 2
levels measured at least 1 month apart) compared to baseline
if the Phe baseline level is < 1200 μmol/L; OR
c) sustained blood Phe reduction of at least 50% (At least 2
levels measured at least 1 month apart) compared to baseline
if the Phe baseline level is > 1200 μmol/L;
AND
3. Demonstrated increase of dietary protein tolerance based on target
set between the clinician and patient; AND
4. Clinically meaningful age-appropriate improvement in:

Neurobehavioural or neurocognitive function or
impairment for patients with such impairments as
determined by peer reviewed clinically validated
scales; OR

Demonstrated improvement in Quality of Life using
peer reviewed validated scales; AND
5. Managed by a physician specialized in metabolic/biochemical
diseases.
Dosage: Up to a maximum of 20 mg/kg per day.
Approval Duration: 1 year
Pregnant Patients:
No renewals will be considered.
Note: Patients enrolled in sapropterin clinical trials PKU 015, PKU 016 and Kuvan
Assistance Program would have to meet provincial coverage criteria in order to
quality for funding.
1. Coverage for Fabry Disease
Saskatchewan participates in the Canadian Fabry Disease Initiative (CFDI).
Prescribers, pharmacists or patients with questions regarding coverage of enzyme
replacement therapy for the treatment and management of Fabry Disease are asked
to call the Drug Plan and Extended Benefits Branch for more information.